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Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3
BRIEF
REPORTS
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3 Anne Bouloc, MD, PhD,a Pascal Joly, MD, PhD,c Estelle Saint-Leger, MD,a Janine Wechsler, MD,b Jean-Claude Roujeau, MD,a and Jean Revuz, MDa Créteil and Rouen, France A patient with follicular B-cell lymphoma presented with erythroderma associated with cutaneous and mucosal blisters. Histologic and direct immunofluorescence analysis of lesional skin showed a typical pattern of paraneoplastic pemphigus (PNP). Interestingly, indirect immunofluorescence on rat bladder was negative and immunoblot analysis of the patient’s serum on epidermal extracts demonstrated antiepidermal antibodies that only recognized the pemphigus vulgaris antigen desmoglein 3, with no antibodies directed against the different proteins of the plakin family. To our knowledge this has never been reported in the literature. It exemplifies the overlap between pemphigus vulgaris and PNP and the pathogenic role of antidesmoglein 3 antibodies in PNP. Moreover, it underscores the need to consider clinical, histologic, and immunologic features for the diagnosis of PNP. (J Am Acad Dermatol 2000;43:714-7.)
P
araneoplastic pemphigus (PNP) has been established as a newly recognized but distinctive mucocutaneous autoimmune disease.1,2 It has characteristic clinical, histologic, and immunopathologic features, consistent between affected persons, and is associated mostly with lymphoproliferative disorders. We describe a patient with lichen planus–like PNP whose serum only recognized the 130-kd antigen of pemphigus vulgaris (PV) by immunoblotting.
CASE REPORT A 59-year-old white man was referred for erythroderma associated with bullous cutaneous and mucosal lesions. The patient had been followed up for 9 years for follicular small B-cell lymphoma. He had had radiotherapy and had been treated by 3 different chemotherapy regimens. A trial of fludarabine was performed because of the persistence of cervical and abdominal lymphadenopathy. Cutaneous lesions appeared 3 weeks after the fourth course and were followed 1 month later by mucosal lesions. Clinical examination at admission revealed a lichenoid erythroderma associated with two bullae on the flank,
From the Departments of Dermatologya and Pathology,b Hôpital Henri-Mondor, Créteil, and the Department of Dermatology and INSERM U 519, Hôpital Charles Nicolle, Rouen.c Reprints not available from authors. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/54/107750 doi:10.1067/mjd.2000.107750
714
ulcerations of the neck and upper part of the chest, conjunctival vesicles, as well as jugal, gingival, labial, and anal erosions (Figs 1-3). There was no Nikolsky sign. No peripheral lymphadenopathy was found. Histologic examination of a bullous skin lesion showed a subepidermal bulla associated with intraepidermal separation, acantholytic cells, and keratinocyte necrosis (Fig 4). Examination of lichenoid peribullous skin revealed an inflammatory infiltrate of small lymphocytes along the dermoepidermal junction with a few colloid bodies. Direct immunofluorescence exhibited deposits of IgG and C3 on the keratinocyte plasma membrane as well as granular deposits of C3 on the dermoepidermal junction. Indirect immunofluorescence was negative on rat esophagus and rat bladder and showed low titers of anti-epidermal cell surface (ECS) antibodies (1:40) on normal human skin. Immunoblot analysis of the patient’s serum revealed the presence of IgG1 antibodies directed against a 130-kd band, which comigrated with the desmoglein 3 band recognized by a control PV serum (Fig 5). As positive control experiments, sera from 22 patients with clinically and histologically well-characterized PNP were immunoblotted on the same epidermal extracts. All 22 sera recognized one or more bands of the PNP complex; 18 sera identified the 190-kd and/or the 210-kd band of envoplakin and periplakin, and 9 sera labeled the 250-kd band of desmoplakin I. The patient was treated by systemic and topical corticosteroids, which resulted in cutaneous and mucosal improvement. A computed tomographic scan of the abdomen
J AM ACAD DERMATOL VOLUME 43, NUMBER 4
Brief reports 715
Fig 2. Labial erosions and erythema of the face.
Fig 1. Eruption of the trunk and limbs with ulcerations of the neck.
showed large lymphadenopathies. The patient was treated by another chemotherapy regimen (CHOP) and died of pneumomonopathy.
DISCUSSION PNP is a new type of pemphigus defined by Anhalt et al1 on the basis of clinical, histologic, and immunologic criteria. Serum autoantibodies are directed against an antigen complex mainly composed of proteins of the plakin family. Desmoplakin I and bullous pemphigoid antigen 1 correspond respectively to the 250-kd and the 230-kd antigens.1 The 210-kd antigen was found to be a doublet corresponding to desmoplakin II and to envoplakin.1,3 The 190-kd antigen was shown to be periplakin.4 The 170-kd antigen has yet to be identified. Other antibodies directed against plectin, another member of the plakin family, have recently been demonstrated in cases of PNP.5 The clinical presentation of our patient was characteristic of PNP because he had follicular B-cell lymphoma and he presented with erythroderma associated with bullous lesions. Five cases of lichen pemphigoides–like PNP have been described in the literature.6-10 The triggering by radiotherapy and the
Fig 3. Eruption of the back.
predominance of the lesions on irradiated zones have been previously reported in PNP.11 Although fludarabine has been implicated in a case of PNP,12 it is unlikely that our case corresponds to a druginduced lichenoid reaction since cutaneous lesions occurred 21 days after the fourth course of the drug. Similarly, the patient had not been transfused or had a graft, which eliminates a lichenoid graft-versus-
716 Brief reports
J AM ACAD DERMATOL OCTOBER 2000
Fig 4. Photomicrograph shows suprabasal separation lined by acantholytic cells associated with foci of necrotic cells. Nonspecific inflammation within papillary dermis. (Hematoxylin-eosin stain; original magnification ×125.)
host disease. Finally, the histologic and immunologic features were different from those of the recently reported cases of erythrodermic lichenoid bullous pemphigoid.13 Histologic characteristics were typical of PNP because they associated subepidermal and intraepidermal bullae, keratinocyte necrosis, and acantholytic cells. Moreover, the deposition of IgG and C3 on ECS and the granular deposits of C3 along the basement membrane zone by direct immunofluorescence was also typical of PNP. Immunoblot analysis of the patient’s serum only detected autoantibodies directed against the PV antigen desmoglein 3, whereas the autoantibody populations directed against the different proteins of the plakin family (envoplakin, periplakin, desmoplakin I and II, BPAg1), which are characteristic of PNP, could not be detected either by immunoblotting or by indirect immunofluorescence analysis on rat bladder, which have been previously reported to be sensitive and reliable methods for the diagnosis of PNP.14-16 It is unlikely that the patient’s serum contained antiplakin antibodies because our sensitive immunoblotting assay permitted us to clearly identify the presence of antiplakin antibodies in 100% of the 22 PNP sera that were tested as positive control experiments. Similarly, Hashimoto et al,16 testing 6 PNP sera by both immunoprecipitation and immunoblotting, demonstrated the presence of antiplakin antibodies by immunoblot in all PNP sera. We initially reported the presence of antidesmoglein 3 antibodies in PNP sera, which has been confirmed by others.17-19 However, these autoantibodies are usually associated with antibodies directed against proteins of the plakin family, especially envoplakin and periplakin.17-20
Fig 5. Immunoblot analysis of the patient’s serum, using human epidermis as the extract. Lane A: Murine antidesmoglein 1 monoclonal antibody (160 kd); lanes B, C, and D: sera from 3 patients with PNP tested as positive control experiments (250, 210, 190, 130 kd); lane E: serum from our patient that recognized 130-kd band comigrating with the band labeled by control PV serum (lane F); lane G: control serum from healthy patient.
The present case raises the question of the overlap between PV and PNP. This has been previously suggested by the overlapping distribution of autoantibody specificities between PV and PNP.17 Indeed, most if not all PNP sera recognize desmoglein 3. Amagai et al19 recently showed that anti-desmoglein 3 antibodies could be consistently found in 25 PNP sera by enzyme-linked immunosorbent assays and demonstrated their pathogenic properties because the injection of anti-desmoglein 3 antibodies induces acantholysis in the neonatal BALB/c mouse model. On the other hand, antiplakin antibodies are not specific for PNP. Indeed, antibodies to desmoplakins I and II have been identified by immunoblotting and immunoprecipitation in sera from patients with nonneoplastic pemphigus foliaceus.21 Similarly, sera from sporadic as well as endemic Tunisian pemphigus foliaceus patients were shown to react with a 180- to 190kd antigen immunolocalized to the desmosomal plaque.22-24 Finally, some pemphigus vulgaris sera have been demonstrated to contain anti-180- to 190kd antibodies that react with both intracellular and extracellular structures of desmosomes.23 Although the immunoblotting profile of our patient was that of PV, such a diagnosis cannot explain the clinical and histologic features of our
J AM ACAD DERMATOL VOLUME 43, NUMBER 4
patient, since the occurrence in a patient with follicular B-cell lymphoma, of erythroderma with a histologic picture of subepidermal and intraepidermal cleavage, keratinocyte necrosis, and lichenoid infiltrate, associated with dual deposits of IgG and C3 on ECS and granular deposits of C3 on the basement membrane zone, are characteristic features of PNP and have never been reported in PV. Overall, this case report reinforces the concept of an overlap between PV and PNP and underscores the need to consider clinical, histologic, and immunologic features for the diagnosis of PNP. REFERENCES 1. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus: an auto-immune disease associated with neoplasis. N Engl J Med 1990;323:1729-35. 2. Anhalt GJ. Paraneoplastic pemphigus. Adv Dermatol 1997;12:797. 3. Kim SC, Kwon YD, Lee IJ, Chang SN, Lee TG. cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. J Invest Dermatol 1997;109:365-9. 4. Mahoney MG, Aho S, Uitto J, Stanley JR. The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol 1998; 111:308-13. 5. Proby C, Fujii Y, Owaribe K, Nishikawa T, Amagai M. Human autoantibodies against HD1/Plectin in paraneoplastic pemphigus. J Invest Dermatol 1999;112:153-6. 6. Camisa C, Helm TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol 1993;129: 883-5. 7. Stevens SR, Griffiths CEM, Anhalt GJ, Cooper KD. Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption. Arch Dermatol 1993;129:866-9. 8. Jansen T, Plewig G, Anhalt GJ. Paraneoplastic pemphigus with clinical features of erosive lichen planus associated with Castleman’s tumor. Dermatology 1995;190:245-50. 9. Krunic AL, Kokai D, Bacetic B, et al. Retroperitoneal round-cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoides-like eruption. Int J Dermatol 1997;36:526-9. 10. Passeron T, Bahadoran P, Lacour JP, et al. Paraneoplastic pemphigus presenting as erosive lichen planus. Br J Dermatol 1999; 140:552-3. 11. Lee MS, Kossard S, Ho KK, Barnetson RS, Ravich RB. Paraneoplastic pemphigus triggered by radiotherapy. Australas J Dermatol 1995;36:206-10.
Brief reports 717
12. Barzabachi A, Bachelez H, Dehen L, Delmer A, Zittoun R, Dubertret L. Lethal paraneoplastic pemphigus following treatment of chronic lymphocyte leukaemia with fludarabine. Ann Oncol 1995;6:730-1. 13. Joly P, Tanasescu S, Wolkenstein P, et al. Lichenoid erythrodermic bullous pemphigoid of the African patient. J Am Acad Dermatol 1998;39:691-7. 14. Liu AY,Valenzuela R, Helm TN, Camisa C, Melton AL, Bergfeld WF. Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. J Am Acad Dermatol 1993;28:696-9. 15. Helou J, Allbritton J, Anhalt GJ. Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol 1995;32:441-7. 16. Hashimoto T, Amagai M, Watanabe K, et al. Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol 1995;104:829-34. 17. Joly P, Thomine E, Gilbert D, et al. Overlapping distribution of autoantibody specificities between pemphigus vulgaris and paraneoplastic pemphigus. J Invest Dermatol 1994;103:65-72. 18. Hashimoto T, Amagai M, Ning W, et al. Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. J Dermatol Sci 1998;17:132-9. 19. Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest 1998;102:775-82. 20. Richard C, Courville Ph, Thomine E, et al. Pemphigus et cancer: étude des caractéristiques cliniques, histologiques et immunologiques d’une cohorte de 28 malades. Ann Dermatol Venereol 1997;124(Suppl):S29. 21. Jiao D, Bystryn JC. Antibodies to desmoplakin in a patient with pemphigus foliaceous. J Eur Acad Dermatol Venereol 1998;11: 169-72. 22. Ghohestani R, Joly P, Gilbert D, et al Autoantibody formation against a 190-kDa antigen of the desmosomal plaque in pemphigus foliaceus. Br J Dermatol 1997;137:774-9. 23. Joly P, Gilbert D, Thomine E, et al. Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus. J Invest Dermatol 1997;108:469-75. 24. Joly P, Mokhtar I, Gilbert D, et al. Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus. Br J Dermatol 1999;140:44-9.
REPORTS
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3 Anne Bouloc, MD, PhD,a Pascal Joly, MD, PhD,c Estelle Saint-Leger, MD,a Janine Wechsler, MD,b Jean-Claude Roujeau, MD,a and Jean Revuz, MDa Créteil and Rouen, France A patient with follicular B-cell lymphoma presented with erythroderma associated with cutaneous and mucosal blisters. Histologic and direct immunofluorescence analysis of lesional skin showed a typical pattern of paraneoplastic pemphigus (PNP). Interestingly, indirect immunofluorescence on rat bladder was negative and immunoblot analysis of the patient’s serum on epidermal extracts demonstrated antiepidermal antibodies that only recognized the pemphigus vulgaris antigen desmoglein 3, with no antibodies directed against the different proteins of the plakin family. To our knowledge this has never been reported in the literature. It exemplifies the overlap between pemphigus vulgaris and PNP and the pathogenic role of antidesmoglein 3 antibodies in PNP. Moreover, it underscores the need to consider clinical, histologic, and immunologic features for the diagnosis of PNP. (J Am Acad Dermatol 2000;43:714-7.)
P
araneoplastic pemphigus (PNP) has been established as a newly recognized but distinctive mucocutaneous autoimmune disease.1,2 It has characteristic clinical, histologic, and immunopathologic features, consistent between affected persons, and is associated mostly with lymphoproliferative disorders. We describe a patient with lichen planus–like PNP whose serum only recognized the 130-kd antigen of pemphigus vulgaris (PV) by immunoblotting.
CASE REPORT A 59-year-old white man was referred for erythroderma associated with bullous cutaneous and mucosal lesions. The patient had been followed up for 9 years for follicular small B-cell lymphoma. He had had radiotherapy and had been treated by 3 different chemotherapy regimens. A trial of fludarabine was performed because of the persistence of cervical and abdominal lymphadenopathy. Cutaneous lesions appeared 3 weeks after the fourth course and were followed 1 month later by mucosal lesions. Clinical examination at admission revealed a lichenoid erythroderma associated with two bullae on the flank,
From the Departments of Dermatologya and Pathology,b Hôpital Henri-Mondor, Créteil, and the Department of Dermatology and INSERM U 519, Hôpital Charles Nicolle, Rouen.c Reprints not available from authors. Copyright © 2000 by the American Academy of Dermatology, Inc. 0190-9622/2000/$12.00 + 0 16/54/107750 doi:10.1067/mjd.2000.107750
714
ulcerations of the neck and upper part of the chest, conjunctival vesicles, as well as jugal, gingival, labial, and anal erosions (Figs 1-3). There was no Nikolsky sign. No peripheral lymphadenopathy was found. Histologic examination of a bullous skin lesion showed a subepidermal bulla associated with intraepidermal separation, acantholytic cells, and keratinocyte necrosis (Fig 4). Examination of lichenoid peribullous skin revealed an inflammatory infiltrate of small lymphocytes along the dermoepidermal junction with a few colloid bodies. Direct immunofluorescence exhibited deposits of IgG and C3 on the keratinocyte plasma membrane as well as granular deposits of C3 on the dermoepidermal junction. Indirect immunofluorescence was negative on rat esophagus and rat bladder and showed low titers of anti-epidermal cell surface (ECS) antibodies (1:40) on normal human skin. Immunoblot analysis of the patient’s serum revealed the presence of IgG1 antibodies directed against a 130-kd band, which comigrated with the desmoglein 3 band recognized by a control PV serum (Fig 5). As positive control experiments, sera from 22 patients with clinically and histologically well-characterized PNP were immunoblotted on the same epidermal extracts. All 22 sera recognized one or more bands of the PNP complex; 18 sera identified the 190-kd and/or the 210-kd band of envoplakin and periplakin, and 9 sera labeled the 250-kd band of desmoplakin I. The patient was treated by systemic and topical corticosteroids, which resulted in cutaneous and mucosal improvement. A computed tomographic scan of the abdomen
J AM ACAD DERMATOL VOLUME 43, NUMBER 4
Brief reports 715
Fig 2. Labial erosions and erythema of the face.
Fig 1. Eruption of the trunk and limbs with ulcerations of the neck.
showed large lymphadenopathies. The patient was treated by another chemotherapy regimen (CHOP) and died of pneumomonopathy.
DISCUSSION PNP is a new type of pemphigus defined by Anhalt et al1 on the basis of clinical, histologic, and immunologic criteria. Serum autoantibodies are directed against an antigen complex mainly composed of proteins of the plakin family. Desmoplakin I and bullous pemphigoid antigen 1 correspond respectively to the 250-kd and the 230-kd antigens.1 The 210-kd antigen was found to be a doublet corresponding to desmoplakin II and to envoplakin.1,3 The 190-kd antigen was shown to be periplakin.4 The 170-kd antigen has yet to be identified. Other antibodies directed against plectin, another member of the plakin family, have recently been demonstrated in cases of PNP.5 The clinical presentation of our patient was characteristic of PNP because he had follicular B-cell lymphoma and he presented with erythroderma associated with bullous lesions. Five cases of lichen pemphigoides–like PNP have been described in the literature.6-10 The triggering by radiotherapy and the
Fig 3. Eruption of the back.
predominance of the lesions on irradiated zones have been previously reported in PNP.11 Although fludarabine has been implicated in a case of PNP,12 it is unlikely that our case corresponds to a druginduced lichenoid reaction since cutaneous lesions occurred 21 days after the fourth course of the drug. Similarly, the patient had not been transfused or had a graft, which eliminates a lichenoid graft-versus-
716 Brief reports
J AM ACAD DERMATOL OCTOBER 2000
Fig 4. Photomicrograph shows suprabasal separation lined by acantholytic cells associated with foci of necrotic cells. Nonspecific inflammation within papillary dermis. (Hematoxylin-eosin stain; original magnification ×125.)
host disease. Finally, the histologic and immunologic features were different from those of the recently reported cases of erythrodermic lichenoid bullous pemphigoid.13 Histologic characteristics were typical of PNP because they associated subepidermal and intraepidermal bullae, keratinocyte necrosis, and acantholytic cells. Moreover, the deposition of IgG and C3 on ECS and the granular deposits of C3 along the basement membrane zone by direct immunofluorescence was also typical of PNP. Immunoblot analysis of the patient’s serum only detected autoantibodies directed against the PV antigen desmoglein 3, whereas the autoantibody populations directed against the different proteins of the plakin family (envoplakin, periplakin, desmoplakin I and II, BPAg1), which are characteristic of PNP, could not be detected either by immunoblotting or by indirect immunofluorescence analysis on rat bladder, which have been previously reported to be sensitive and reliable methods for the diagnosis of PNP.14-16 It is unlikely that the patient’s serum contained antiplakin antibodies because our sensitive immunoblotting assay permitted us to clearly identify the presence of antiplakin antibodies in 100% of the 22 PNP sera that were tested as positive control experiments. Similarly, Hashimoto et al,16 testing 6 PNP sera by both immunoprecipitation and immunoblotting, demonstrated the presence of antiplakin antibodies by immunoblot in all PNP sera. We initially reported the presence of antidesmoglein 3 antibodies in PNP sera, which has been confirmed by others.17-19 However, these autoantibodies are usually associated with antibodies directed against proteins of the plakin family, especially envoplakin and periplakin.17-20
Fig 5. Immunoblot analysis of the patient’s serum, using human epidermis as the extract. Lane A: Murine antidesmoglein 1 monoclonal antibody (160 kd); lanes B, C, and D: sera from 3 patients with PNP tested as positive control experiments (250, 210, 190, 130 kd); lane E: serum from our patient that recognized 130-kd band comigrating with the band labeled by control PV serum (lane F); lane G: control serum from healthy patient.
The present case raises the question of the overlap between PV and PNP. This has been previously suggested by the overlapping distribution of autoantibody specificities between PV and PNP.17 Indeed, most if not all PNP sera recognize desmoglein 3. Amagai et al19 recently showed that anti-desmoglein 3 antibodies could be consistently found in 25 PNP sera by enzyme-linked immunosorbent assays and demonstrated their pathogenic properties because the injection of anti-desmoglein 3 antibodies induces acantholysis in the neonatal BALB/c mouse model. On the other hand, antiplakin antibodies are not specific for PNP. Indeed, antibodies to desmoplakins I and II have been identified by immunoblotting and immunoprecipitation in sera from patients with nonneoplastic pemphigus foliaceus.21 Similarly, sera from sporadic as well as endemic Tunisian pemphigus foliaceus patients were shown to react with a 180- to 190kd antigen immunolocalized to the desmosomal plaque.22-24 Finally, some pemphigus vulgaris sera have been demonstrated to contain anti-180- to 190kd antibodies that react with both intracellular and extracellular structures of desmosomes.23 Although the immunoblotting profile of our patient was that of PV, such a diagnosis cannot explain the clinical and histologic features of our
J AM ACAD DERMATOL VOLUME 43, NUMBER 4
patient, since the occurrence in a patient with follicular B-cell lymphoma, of erythroderma with a histologic picture of subepidermal and intraepidermal cleavage, keratinocyte necrosis, and lichenoid infiltrate, associated with dual deposits of IgG and C3 on ECS and granular deposits of C3 on the basement membrane zone, are characteristic features of PNP and have never been reported in PV. Overall, this case report reinforces the concept of an overlap between PV and PNP and underscores the need to consider clinical, histologic, and immunologic features for the diagnosis of PNP. REFERENCES 1. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus: an auto-immune disease associated with neoplasis. N Engl J Med 1990;323:1729-35. 2. Anhalt GJ. Paraneoplastic pemphigus. Adv Dermatol 1997;12:797. 3. Kim SC, Kwon YD, Lee IJ, Chang SN, Lee TG. cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. J Invest Dermatol 1997;109:365-9. 4. Mahoney MG, Aho S, Uitto J, Stanley JR. The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol 1998; 111:308-13. 5. Proby C, Fujii Y, Owaribe K, Nishikawa T, Amagai M. Human autoantibodies against HD1/Plectin in paraneoplastic pemphigus. J Invest Dermatol 1999;112:153-6. 6. Camisa C, Helm TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol 1993;129: 883-5. 7. Stevens SR, Griffiths CEM, Anhalt GJ, Cooper KD. Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption. Arch Dermatol 1993;129:866-9. 8. Jansen T, Plewig G, Anhalt GJ. Paraneoplastic pemphigus with clinical features of erosive lichen planus associated with Castleman’s tumor. Dermatology 1995;190:245-50. 9. Krunic AL, Kokai D, Bacetic B, et al. Retroperitoneal round-cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoides-like eruption. Int J Dermatol 1997;36:526-9. 10. Passeron T, Bahadoran P, Lacour JP, et al. Paraneoplastic pemphigus presenting as erosive lichen planus. Br J Dermatol 1999; 140:552-3. 11. Lee MS, Kossard S, Ho KK, Barnetson RS, Ravich RB. Paraneoplastic pemphigus triggered by radiotherapy. Australas J Dermatol 1995;36:206-10.
Brief reports 717
12. Barzabachi A, Bachelez H, Dehen L, Delmer A, Zittoun R, Dubertret L. Lethal paraneoplastic pemphigus following treatment of chronic lymphocyte leukaemia with fludarabine. Ann Oncol 1995;6:730-1. 13. Joly P, Tanasescu S, Wolkenstein P, et al. Lichenoid erythrodermic bullous pemphigoid of the African patient. J Am Acad Dermatol 1998;39:691-7. 14. Liu AY,Valenzuela R, Helm TN, Camisa C, Melton AL, Bergfeld WF. Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. J Am Acad Dermatol 1993;28:696-9. 15. Helou J, Allbritton J, Anhalt GJ. Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol 1995;32:441-7. 16. Hashimoto T, Amagai M, Watanabe K, et al. Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol 1995;104:829-34. 17. Joly P, Thomine E, Gilbert D, et al. Overlapping distribution of autoantibody specificities between pemphigus vulgaris and paraneoplastic pemphigus. J Invest Dermatol 1994;103:65-72. 18. Hashimoto T, Amagai M, Ning W, et al. Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. J Dermatol Sci 1998;17:132-9. 19. Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest 1998;102:775-82. 20. Richard C, Courville Ph, Thomine E, et al. Pemphigus et cancer: étude des caractéristiques cliniques, histologiques et immunologiques d’une cohorte de 28 malades. Ann Dermatol Venereol 1997;124(Suppl):S29. 21. Jiao D, Bystryn JC. Antibodies to desmoplakin in a patient with pemphigus foliaceous. J Eur Acad Dermatol Venereol 1998;11: 169-72. 22. Ghohestani R, Joly P, Gilbert D, et al Autoantibody formation against a 190-kDa antigen of the desmosomal plaque in pemphigus foliaceus. Br J Dermatol 1997;137:774-9. 23. Joly P, Gilbert D, Thomine E, et al. Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus. J Invest Dermatol 1997;108:469-75. 24. Joly P, Mokhtar I, Gilbert D, et al. Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus. Br J Dermatol 1999;140:44-9.